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中央性 giant 细胞肉芽肿:诺南综合征患者使用地舒单抗的超适应证治疗。

Central giant cell granuloma: Off-label treatment with Denosumab in a patient with Noonan syndrome.

机构信息

Maxillofacial Surgery Department, Hospital Clínico Universitario San Carlos, Madrid 28040, Spain.

Maxillofacial Surgery Department, Hospital Clínico Universitario San Carlos, Madrid 28040, Spain.

出版信息

J Stomatol Oral Maxillofac Surg. 2024 Feb;125(1):101640. doi: 10.1016/j.jormas.2023.101640. Epub 2023 Sep 18.

Abstract

This study aims to describe the utilization of Denosumabࣨ, a human monoclonal antibody against the RANK-L receptor, in a mandibular giant cell granuloma (GCG) with a significant local aggressiveness component that was unresponsive to surgical treatment. We present a case of a 19-year-old male patient diagnosed with Noonan syndrome, who presented a multifocal giant cell granuloma with aggressive behaviour resistant to surgical treatment. Due to the functional and aesthetic implications associated with a surgical procedure, a decision was made to initiate medical treatment using Denosumabࣨ. Throughout the treatment, the patient presented excellent clinical and analytical tolerance, with no reported adverse effects. Surgical intervention remains the preferred approach for GCG. Denosumabࣨ emerges as an alternative, either as neoadjuvant treatment or as definitive therapy for unresectable or resectable tumors associated with significant morbidity. It leads to size stabilization and regression of the tumour stage.

摘要

本研究旨在描述 denosumabࣨ在下颌骨巨细胞肉芽肿(GCG)中的应用,该患者存在明显局部侵袭性成分,对手术治疗无反应。我们报告了一例 19 岁男性患者,诊断为努南综合征,患有多灶性巨细胞肉芽肿,具有侵袭性行为,对手术治疗有抵抗力。由于手术治疗存在功能和美容方面的影响,决定使用 denosumabࣨ进行药物治疗。在整个治疗过程中,患者表现出良好的临床和分析耐受性,没有报告不良反应。手术干预仍然是 GCG 的首选方法。denosumabࣨ作为一种替代方法,无论是作为新辅助治疗还是作为不可切除或可切除肿瘤的确定性治疗方法,均可用于治疗与高发病率相关的肿瘤。它可使肿瘤的大小稳定和消退。

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